Schamburg's Disease Causes Red Spots On The Legs

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Question: I am a woman in my 40s who recently developed red spots on my legs. These began to spread and now cover most of my legs. They do not itch or hurt but are very unsightly.

I went to a dermatologist who told me I have something called Schamburg's disease and there is not much that can be done for it.

Do I have to live with these spots from now on?

Answer: Schamburg's disease is one of several progressive, pigmented purpuric dermatoses. This sounds like a mouthful, but all it means is a skin disorder characterized by the developement of red or purple spots that may become progressively worse with time.

Purpura are small colored spots on the skin caused by collections of red blood cells. These can be caused by any trauma that ruptures the tiny capillaries beneath the skin. They also can be caused by conditions in which the capillaries become unusually fragile or leaky.

A deficiency in the normal clotting factors in the blood can lead to spontaneous bleeding, or bleeding with minimal trauma. Purpura can be distinguished from discoloration caused by enlarged or dilated capillaries under the skin by a simple test.

If one takes a glass microscope slide and presses it over the spot, the spot will blanch and disappear if the blood is in a vessel because the pressure squeezes the blood out of that area of the vessel. With purpura, since the blood cells are not in a vessel, the spots will not blanch.

The cause of Schamburg's disease is not known. It is more common in older patients and is only infrequently seen in patients younger than 40. It seems to be seen more often in people who are on their feet a lot. This is probably because blood pressure in the tiny skin capillaries is highest in the legs because of the effect of gravity on the tissues.

The disease does not appear to be caused by trauma, although once the condition is present, trauma to the skin may cause more spots to form.

The underlying problem seems to be one of an inflammation of the tiny skin capillaries. If a biopsy of the skin is taken, the capillaries look more bent and twisted than normal.

This may affect blood flow through them as the red blood cells try to pass through the tortuous channels. The inflammation of the vessels also makes them ``leaky,'' allowing red blood cells to pass through them into the surrounding tissues.

The course of Schamburg's varies. But typically the spots arise without any apparent cause, spread and increase in number for a period of several weeks and then begin to fade away.

This process may take four to six weeks. The spots usually remain in the lower legs below the knees, but in more severe cases they can extend up the legs and onto the lower torso. The fading of the spots is a result of the absorption of the blood cells by the tissues over time.

Unfortunately, as the spots fade, they may leave a brown pigmentation in the skin.

Red blood cells are red because of the hemoglobin pigment inside them. When hemoglobin is oxygenated, it is bright red, which is why arterial blood - which carries oxygen to the tissues - is bright red.

Hemoglobin is an iron pigment, and when red blood cells break down and are absorbed, the iron pigment can remain behind and stain the skin a brown color. This can happen from any condition in which bleeding into the skin occurs, such as a bruise. This brown color in the skin usually fades. But that can take a year or more, and sometimes it never fades away completely.

There is no known consistently effective treatment for Schamburg's. Vitamin C, about 1,000 mg per day orally, has been beneficial in some people in stabilizing the process.

We know that people with vitamin C deficiency are prone to bleed spontaneously. Because it seems to be an inflammatory process, steroids, which are the most potent anti-inflammatory agents we have, may help.

During the early phase of the disease, when new lesions are forming, a short course of high-dose oral steroids may help.

These are usually given for seven days on a tapering schedule, with the highest dose taken the first day and lower and lower doses taken on subsequent days.

Topical steroids have been used, too. The most effective ones are the stronger prescription formulations, such as Lidex and Halog.

When hyperpigmentation occurs from the brown iron pigment, creams containing chemicals known as hydroquinones can be tried. These are skin lighteners sometimes used for post-inflammatory skin pigmentation, such as from acne, and pigmentation that sometimes occurs with pregnancy and birth-control pills.

Unfortunately, the pigment from purpura tends to be found in the deeper levels of skin, and the hydroquinones are less effective for these.

One final way to try to eliminate brown areas is with the use of a specific laser, the pulsed dye laser, which may selectively target the pigment deposits without damaging the overlying skin.

In the presence of purpura without an obvious cause, it is worth considering having a skin biopsy and hemotologic work-up to be sure some other cause of the spots is not at work. You might ask your dermatologist about this.

If you are on medications, check to see that these do not affect bleeding. Let your doctor know if you are on any herbal remedies or supplements.